Is pots life threatening?

Woman dies 36 times in one year due to rare condition

During each episode Sara experiences rapid palpitations that regularly cause her heart to stop beating and her blood pressure to plummet to what doctors record as clinically dead.

On occasion the attacks can last up to half-an-hour.

Sara’s attacks can last up to half-an-hour

She said: “In reality this was quite a mild attack. As you can see my heart rate is very up.

“Then you see me go limp and the doctors and nurses all crowd round.

“They can’t give me CPR because CPR tires the heart out for no reason so it is pointless. When the heart fills up with blood again is when it begins beating again.

“The blood tends to pool in my legs and the doors need to help feed it back to the heart.”

Sara said it is about four minutes into the video, when they start giving her oxygen and applying canulas, that she clinically dies.

“I can’t have oxygen because it prolongs the attack. In the video here it is before I was properly diagnosed so the doctors have panicked which is why you see them giving me oxygen.

“In fact it is just a case of leaving me there and praying. The only thing they can do is put me on a drip,” she added.

The video was shot by medics assessing Sara at the Royal Hallamshire Hospital in Sheffield, South Yorks, on June 27, 2012.

Sara, from Doncaster, said each time her heart stops medics have to inflict pain on her to shock her into coming back to life.

She said: “When it happens paramedics try and do anything to inflict pain to try and shock me into coming back to life.

“A lot of the time I’ll wake up with big bruises. On one occasion they ripped an acrylic nail off but that still didn’t make me flinch.”

Sara said that she has contacted people with PoTS, but none of those people’s hearts have stopped; on its own, PoTS does not have this effect and is not life threatening.

“They say when you die your hearing is the last thing to go and that’s been my experience.

“After an attack I’ll wake up and my chest will be killing and I’m really tired.”

“I get asked it quite a lot, but there is definitely no bright light. Everything just goes black.

“You can still hear things and there is a voice in my head that is still active and still conscious and it is trying to communicate with those around me. I can remember what people are saying when I black out but have no other awareness of what’s going on.”

CORRECTION: A previous version of this article attributed the attacks suffered by Sara Brautigam solely to the Postural Orthostatic Tachycardia Syndrome (PoTS) from which she suffers. In fact, PoTS on its own does not cause the heart to stop beating, and is not life threatening. Sara’s condition arises from the combination of PoTS and her other medical conditions. We are happy to make this clear, and the text has been amended accordingly.

Though No Cure for POTS, Symptoms Can Often be Effectively Managed

June 11, 2010

Dear Mayo Clinic:

Is it possible to cure postural orthostatic tachycardia syndrome (POTS)? My 15 year old granddaughter has recently been diagnosed with POTS.

Answer:

A cure for POTS doesn’t exist at this time. Fortunately, teenagers — a group commonly affected by POTS — usually grow out of the disorder by the time they reach their early 20s. In the meantime, POTS symptoms can often be effectively managed with a combination of lifestyle and dietary changes, along with medication.

POTS affects the autonomic nervous system, which controls involuntary body functions. In POTS, the nerves that regulate blood flow are out of balance, so blood doesn’t go to the right places at the right time. The result is a variety of symptoms, including postural orthostatic tachycardia — an overly rapid heartbeat that occurs when shifting position from lying down to standing up.

A person’s heart rate is usually 70 or 80 beats per minute when lying down. Normally, the heart rate rises by about 10 to 15 beats per minute upon standing. For people with POTS, the heart rate goes up considerably higher when they stand, often by 30 to 50 beats per minute or more, which may lead to dizziness and fainting.

Other POTS symptoms include chronic fatigue; headaches or other types of chronic pain; and digestive problems, such as nausea and cramping. These symptoms may vary considerably from one person to another.

POTS frequently begins in the early adolescent years, often between 12 and 15. Onset typically follows a serious injury or illness — often a viral infection accompanied by a high fever. Although it’s not clear why POTS begins this way, it seems to be associated with the hormonal changes of early puberty, coupled with the way the body is affected by a severe illness or injury.

The good news is that, although POTS is a chronic condition, about 80 percent of teenagers grow out of it once they reach the end of their teenage years, when the body changes of puberty are finished. Most of the time, POTS symptoms fade away by age 20. Until recovery takes place, treatment can be helpful.

In people who have POTS, the blood vessels are too relaxed. Extra fluid is needed to fill the vessels and allow blood to flow properly. But drinking extra fluids alone isn’t enough. Increased salt intake is necessary to hold the fluid in the blood vessels. It’s also important for people with POTS to avoid caffeine and alcohol.

What will help most, though, is regular exercise. Engaging in daily, moderate, aerobic exercise in an upright position — walking or biking, for example — not only improves fitness and increases energy, it also works to retrain the autonomic nervous system to regulate blood flow correctly. The key is to start small and progress slowly to about 30 minutes of exercise each day.

A variety of medications are also available to treat POTS. They include drugs that block blood vessel relaxation, help tighten blood vessels and smooth nerve transmission.

In addition, many teenagers with POTS benefit from professional psychological counseling to help them deal with anxiety or other complicating factors, manage the depression that can result from the effects of POTS, or simply cope with a chronic condition.

Because POTS is a complicated disorder that manifests itself in a variety of ways, patients with POTS may need to take advantage of various treatment options. At times, it can be challenging to find a treatment regime that successfully controls POTS symptoms. If someone hasn’t responded well to initial treatment, it’s important to continue medical care and work with a doctor familiar with POTS who can customize care to the individual’s needs.

Although it may be debilitating in some cases, POTS often can be managed so the person affected can return to an active lifestyle. For most, POTS is a disorder that will eventually go away. Optimism is appropriate!

— Phil Fischer, M.D., Pediatrics, Mayo Clinic, Rochester, Minn.

POTS: A Mysterious Syndrome That Can Turn Your Life Upside Down

Imagine you’ve just given birth to your second child, but you’re unable to care for your baby because you can’t stand or walk without the fear of fainting. You’re nauseous, vomiting, and experiencing headaches and shortness of breath. You might even become incontinent. Your brain is so foggy that you can’t think straight, you can’t sleep, and you’re so exhausted that you can’t get out of bed. You’re actually afraid you could be dying.

Meanwhile, others must take care of your newborn because you’re just not strong enough to hold him, and you’re terrified you’ll drop him if you do — all because of your mystery illness.

Wendy Baruchowitz, 44, of Rye, New York, survived this nightmare scenario and came through it a thoroughly changed woman. She now dedicates much of her time to helping others who have the mysterious condition known as POTS, or postural orthostatic tachycardia syndrome.

What Is POTS?

POTS is an unusual condition in which your heart rate speeds up by 30 or more beats per minute with little or no change in blood pressure. Such an increase in heart rate may mean that your cardiovascular system is working as hard as it can to maintain blood pressure and blood flow to your brain. According to the Rare Diseases Clinical Research Network, POTS affects about 500,000 Americans, most of whom are women under age 35.

“POTS is typically not life-threatening, but it is life-altering,” says Svetlana Blitshteyn, MD, clinical assistant professor at the University of Buffalo School of Medicine and Biomedical Sciences and director of the Dysautonomia Clinic in Williamsville, New York. It’s also characterized by lightheadedness when standing, notes the National Institute of Neurological Disorders and Stroke. POTS may have multiple causes, Dr. Blitshteyn says.

Dysautonomia International, a nonprofit organization that provides support for and educational materials about POTS, has found that it tends to occur in younger, premenopausal women like Baruchowitz, according to an ongoing patient survey.

Before she had POTS, Baruchowitz was a busy, active mother who worked at an advertising agency in New York City. “I lived what most people would call a normal life — I was a mom, I commuted to work every day, I traveled, and I attended parties and social events with friends and family without giving it a second thought. I took things for granted, as I had no idea what lay ahead on my horizon,” she recalls.

POTS Symptoms, but No Diagnosis

While she was pregnant with her second son, Baruchowitz, then 39, experienced minor complications and her doctors ordered bed rest for several months as a precaution. Just a week before her due date, she was thrilled to get the green light to move around again. But when she stood up for the first time, she immediately knew something was wrong. “I could feel the blood draining from my head and pooling in my feet. My heart was racing. I was dizzy and nauseous,” she says.

Baruchowitz asked her husband, Mitch, to take her pulse, which was 160 — about twice what it should be for a woman nearing her due date.

She and her husband explained away her symptoms as simply the result of being out of shape after long weeks of bed rest. But a week after little Blake was born, they knew something far worse was happening. “I had lost the ability to function. I was petrified and became despondent,” recalls Baruchowitz. She was also desperate for answers.

Her desperation drove her out of bed, and over the next few months, she saw dozens of doctors — all of whom dismissed her symptoms as nothing serious. “Neurologists, internists, and cardiologists all told me that I was suffering from postpartum depression or that I had anxiety from the birth of my son,” she recalls.

The solutions the doctors offered insulted her: psychological counseling or anti-anxiety medication, as if her terrifying symptoms were all in her head.

A POTS Protocol for Gradual Recovery

After an exhaustive search for a diagnosis that made sense to her, she finally found Blair Grubb, MD, director of both the Clinical Electrophysiology Program and the Clinical Autonomic Disorders & Syncope Center at the University of Toledo Medical Center in Ohio. Dr. Grubb, a POTS expert, was finally able to correctly diagnose Baruchowitz and created an individualized POTS protocol for her that would ultimately restore her to a semblance of her former life.

That protocol, which might not work for everyone who has the condition, includes daily exercise, a high-salt diet, and drinking 60 to 80 ounces (2 to 2.5 quarts) of water every day.

The main thing Baruchowitz wants everyone with this baffling condition to understand is that “You can regain control of your body and significantly improve over time,” she says. When she first started swimming, she was barely able to eke out one lap in the pool. But she worked with a personal trainer, swam every single day, and over a few months progressed to swimming 30 minutes a day. Only then was she ready to exercise on solid ground. Swimming is a perfect exercise to begin with because you’re horizontal, and swimming defies gravity — the enemy to a POTS patient, she notes.

Strengthened by her swimming, Baruchowitz then began working out on the treadmill, at first walking just one to three minutes a day. “Today, I can walk/run two miles a day and I also walk 11,000-plus steps a day — roughly 5.5 miles,” she says proudly. Plus, she weight trains three times a week. Weight training is particularly important for people with POTS, because building muscle mass is critical for pumping blood back to the heart, which helps prevent fainting.

Thankfully for Baruchowitz and others who face the challenges of POTS, there’s more research interest and knowledge about POTS than in the past, says Blitshteyn. “There is some evidence that POTS may have an autoimmune basis in some patients,” she says, noting that certain antibodies — alpha 1 adrenergic receptor and beta 1 andrenergic receptors — have been found in some people with POTS. Researchers are currently studying 400 patients to see if POTS is, in fact, an autoimmune disease, says Blitshteyn.

Genetic factors may also play a role. “The two most important are excessive joint flexibility, often from a benign form of Ehlers-Danlos syndrome, a type of inherited excess joint laxity,” explains Louis H. Weimer, MD, professor of neurology and codirector of the Columbia Neuropathy Research Center at the Columbia University Medical Center in New York City. Or it could be triggered after an acute viral infection such as the flu, he notes.

POTS patients commonly report that they’ve had some kind of bacterial or viral infection, such as mononucleosis, flu, or gastroenteritis, adds Blitshteyn. “They appear unable to recover and continue to experience fatigue, flu-like illness, dizziness, nausea, and headaches,” she adds.

Patient Advocates for POTS Awareness

Baruchowitz created POTS Take A Stand as a platform to raise funds and awareness for POTS research. Partnering with Dysautonomia International, she helped organize a one-mile walk last October in Rye, New York. The event raised $20,000 for Dysautonomia International’s POTS Research Fund. Baruchowitz recently joined the group’s patient advisory board, a vantage point that allows her to connect with and help patients, parents, and caregivers affected by POTS.

Since then, she says, countless people have reached out for support in finding doctors, advice about protocols, exercise regimens, and dietary tips. “It’s been so gratifying helping these people, especially since I know how difficult life can be living with POTS,” she says.

IS POTS AN AUTOIMMUNE DISEASE?

Big news this week in POTS research! Researchers from the University of Oklahoma and Vanderbilt University have identified evidence of adrenergic receptor autoantibodies in a small group of POTS patients, suggesting that POTS may be an autoimmune condition in these patients. The study was published in the Journal of the American Heart Association (JAHA). JAHA is an official journal of the American Heart Association, so this is great news for POTS awareness!

To help patients better understand what this means, Dr. David Kem from the University of Oklahoma Health Sciences Center has kindly provided Dysautonomia International with a patient friendly explanation of this complex research. Before we get to Dr. Kem’s explanation, let’s go over the basics of adrenergic receptors and autoantibodies.

Adrenergic receptors are present on the surface of cells in many different parts of the body, including the heart, blood vessels, nerves, brain, lungs, bladder, gastrointestinal tract and elsewhere. There are two main types of adrenergic receptors in the body – alpha adrenergic receptors and beta adrenergic receptors. Within the alpha and beta types, there are many different subtypes (alpha-1, alpha-2A, alpha-2B, alpha-2C, beta-1, beta-2, etc.)

Think of adrenergic receptors like a TV antenna (if you are old enough to remember when TVs had antennas!). If the TV antenna picks up a signal, it transmits a message across the screen. In adrenergic receptors the “signals” are chemicals present in the body called catecholamines (primarily epinephrine and norepinephrine). The “message” is what the catecholamine tells the receptor to do. For example, constrict a blood vessel or make the heart beat faster.

Image of an adrenergic receptor, which is stimulated by catecholamines.

Antibodies are proteins created by your own immune system to protect you from pathogens, like bacteria and viruses. The human immune system can make more than 1 trillion different antibodies, each one meant to protect us from a different pathogen. Unfortunately, sometimes the antibody formation process goes awry, and the antibodies created by your immune system can turn against your own cells. These trouble-making antibodies are called autoantibodies. Autoantibodies can attack, damage or interfere with the functioning of healthy tissues and cells in your body.

Now that we all know what adrenergic receptors and autoantibodies are, here is what Dr. Kem has to say about the adrenergic receptor autoantibodies recently found in POTS patients:

POTS occurs frequently, but not exclusively, in younger females and its onset is occasionally preceded by or associated with a viral-like illness. It is more than a minor annoyance for most patients and leads to significant life changes and limitations in normal life. Our present study (Autoimmune Basis for Postural Tachycardia Syndrome) has produced data supporting the idea that production of autoantibodies, circulating proteins that normally fight such infections, have instead interacted with critical site(s) on specialized cell membrane proteins which alter their normal cell function.

These autoantibodies interfere with the system which controls the ability of blood vessels to constrict, which is needed to prevent a drop of blood pressure as a person stands. In POTS patients, this inadequate response to standing leads to a generalized increase of activity in the body’s sympathetic nerve system, which frequently normalizes the blood pressure. This increased nerve activity, however, increases the heart rate which is a prominent symptom in POTS.

We have also discovered a second group of autoantibodies in some POTS patients which directly increase the heart rate.

The combination of these two autoantibodies appears to cause the abnormal heart rate response observed in all 14 POTS patients we have tested to date for these autoantibodies. We have previously identified similar autoantibodies in individuals diagnosed with idiopathic orthostatic hypotension (Editor’s note: see Agnostic Autoantobodies as Vasodilators in Orthostatic Hypotension: A New Mechanism and Autoantibody Activation of Beta-Adrenergic and Muscarinic Receptors Contributes to an “Autoimmune” Orthostatic Hypotension).

These autoantibodies may explain why beta blockers aren’t always effective in treating the tachycardia seen in POTS, since beta blockers fail to completely block autoantibody activity on their protein receptor and they fail to alter the partial blockade of the autoantibodies on the arteriole blood vessels that initiate the orthostatic problem.

Confirmation of our findings will require testing a larger group of POTS patients for these autoantibodies. We hope to eventually develop treatments to block these autoantibodies, without blocking the target receptor proteins at the cell surface at the same time. Such agents are in development and within a few years may be applicable in POTS. This approach may prove useful in several other diseases which are caused by similar autoantibodies.

Please note that Dr. Kem and the other researchers involved are not able to test patient blood samples for these autoantibodies outside of a research setting at this time. There are very strict federal laws that prohibit them from doing so. If such a test becomes available to the public, Dysautonomia International will be shouting it from the roof tops. Imagine that – a blood test to help diagnose POTS ? We’re looking forward to it, but there is much work to be done.

Dysautonomia International is committed to funding additional research in this area as quickly as possible. We are optimistic that this will lead to a better understanding of POTS, better ways to diagnose it, and most importantly, better ways to treat it.

If you would like to support the next phase of this exciting new research, please consider making a donation to Dysautonomia International today. You can make a difference in the lives of millions of people around the world living with POTS!

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GP Guide

WHAT IS POSTURAL TACHYCARDIA SYNDROME?

PoTS was characterised in 1993, but previously existed under various other names including irritable heart, soldier’s heart and idiopathic orthostatic intolerance. It is a heterogeneous group of disorders sharing similar characteristics. On assuming upright posture, there is an excessive increase in heart rate associated with symptoms of orthostatic intolerance and sympathetic over-activity. There is brain hypoperfusion, usually in the absence of hypotension.
When humans adopt upright posture, approximately 500ml of blood drops into the abdominal cavity and limbs. A normal autonomic nervous system responds with immediate peripheral vasoconstriction and an increase in heart rate of up to 20bpm.
In PoTS, it is considered that vasoconstriction is inadequate, resulting in pooling of blood, relative hypovolaemia and reduced venous return to the heart. Heart rate, inotropic status and, in some patients, catecholamine levels increase further to compensate. Dizziness and syncope can occur in the presence of normal BP; in fact some patients with PoTS have a hypertensive response to standing.

HOW COMMON IS PoTS?

The incidence in the UK is unknown. However, it is probably under-diagnosed due to lack of awareness and non-specific symptomatology. It is five times more common in women and tends to affect people age 15 to 50.

SYMPTOMS OF PoTS

  • Dizziness
  • GI upset
  • Syncope / pre-syncope
  • Sweating
  • Orthostatic headache
  • Nausea
  • Fatigue
  • Insomnia
  • Poor memory
  • Weakness
  • Poor concentration
  • Visual greying or blurring
  • Sense of anxiety
  • Acrocyanosis (purplish hands/feet)
  • Exercise intolerance
  • Palpitations (tachycardia / ectopics)
  • Tremulousness
  • Neck/shoulder pain (muscle ischaemia)

Patients may have some or all of the above symptoms. Most patients experience light-headedness, but 30% have blackouts. Symptoms tend to be worse on standing or prolonged sitting and exacerbated by heat, food and alcohol. Life expectancy is thought to be unaffected, but levels of disability can be equivalent to that found in congestive heart failure and COPD.

WHAT CAUSES PoTS?

PRIMARY PoTS

  • Abrupt onset may follow infection, pregnancy, surgery, immunisation or trauma. There is some evidence of auto-immune aetiology.
  • Developmental’ PoTS affects teenagers (gradual onset around age 14 with symptoms peaking age 16) and 80% resolve within a few years.
  • Hyperadrenergic PoTS – in some, a genetic defect has been identified

SECONDARY PoTS

  • Deconditioning (e.g. after prolonged bed rest following illness)
  • Joint Hypermobility Syndrome (now considered by most authorities as indistinguishable from, if not identical to, the Ehlers-Danlos-hypermobility type, formerly known as EDS type III); an inherited multi-system connective tissue disorder. See hypermobility.org/
  • Other medical conditions – diabetes, amyloidosis, sarcoidosis, SLE *
  • Poisons – alcohol, heavy metals *
  • Cancer or chemotherapy

HOW IS IT DIAGNOSED?

Diagnosis is usually made following a tilt table test. After resting, the patient is tilted head up by around 60 degrees for up to 45 minutes. In PoTS, heart rate increases by at least 30 bpm (40bpm in teenagers) or to >120 bpm. Symptoms are similar to those of hypotension and there is reduced cerebral perfusion, but by definition, blood pressure does not necessarily drop. However, there is an overlap with neurally mediated hypotension and 40% also have a drop in blood pressure.

A stand test can also be used to diagnose PoTS. Pulse rate / BP can be measured supine then after 2, 5 and10 minutes of standing still. Look for acrocyanosis (puffiness and purplish discolouration of feet/hands). A sustained increase in heart rate of >30 bpm suggests PoTS. Stop if patient becomes very symptomatic. Some patients may faint during this test.

Plasma noradrenaline levels are often elevated in the upright position in PoTS.
Other pathology must be excluded and underlying causes of PoTS considered.
Depending on history and symptoms, tests may include FBC, biochemical profile, calcium, thyroid function, serum cortisol, catecholamine levels, urine HIAAs, ECG, 24 hour BP, ECG and EEG.
DIAGNOSTIC CRITERIA FOR PoTS

  1. Sustained heart rate increase of 30 beats per minute or more within 10 minutes of standing or head up tilt (40 bpm age 12-19) in the absence of orthostatic hypotension.
  2. Standing heart rate is often 120 beats per minute or more within 10 minutes of standing or head up tilt
  3. Orthostatic tachycardia may be accompanied by symptoms of cerebral hypo-perfusion and autonomic over-activity that are relieved by recumbence.
  4. Criteria not applicable for low resting heart rate.

MANAGEMENT

PHYSIOLOGICAL

  • Increased fluid (2-2.5 litres/day) and salt intake (2 to 4g ie up to 2/3 level teaspoon, Not in hyperadrenergic PoTS) * – increases blood volume
  • Graduated exercise programme – swimming is ideal – improves calf muscle pump
  • Small frequent meals low in refined carbohydrates -reduces diversion of blood to gut
  • Elevate head of bed * – increases morning blood volume
  • Avoid prolonged standing and sitting * – reduces venous pooling
  • Avoid heat and alcohol * – reduce vasodilatation
  • Support tights – class 3, waist high * – reduce venous pooling
  • Postural manoeuvres to avoid syncope eg elevate legs, cross legs and clench thighs and buttocks, clench a fist, rock up and down on tiptoes *- improves venous return

DRUG THERAPY

Stop drugs that may cause orthostatic intolerance.
Considered drug therapy if 3 – 6 months of physiological measures are ineffective.
Drugs used in PoTS are all unlicensed for this indication.
Some hospitals offer a shared care agreement.

  • Midodrine-Alpha -1 agonist / vasoconstrictor
  • Beta blocker-Reduce heart rate and improves diastolic filling
  • Ivabradine-Reduce heart rate and improves diastolic filling
  • SSRIs / SNRIs-Affects central control of HR and BP
  • Clonidine- Alpha-2 agonist / centrally sympatholytic
  • Desmopressin- Increases blood volume
  • Methylphenidate-Vasoconstricts
  • Erythropoeitin- Increase red cell mass/vasoconstricts
  • Octreotide-Splanchnic vasoconstrictor
  • Pyridostigmine-Facilitates ganglionic neural transmission

PSYCHOLOGICAL SUPPORT

CBT to help patients adjust to long-term illness and symptoms

WHY IS PoTS UNDERDIAGNOSED?

  • Patients can repeatedly present with a multitude of symptoms including anxiety, often without clinical findings – typical ‘heart-sink’ patients.
  • Patients may not recognise the significance of symptoms or be reluctant to divulge them for fear of the clinician’s response.
  • Blood pressure and pulse recordings are usually taken with patients seated when recordings may be normal.
  • There is little knowledge of PoTS within the medical community.
  • It is often misdiagnosed as anxiety, panic attacks, vasovagal syncope, chronic fatigue syndrome or inappropriate sinus tachycardia.

WHY IS THIS IMPORTANT?

  • Diagnosis of PoTS is commonly delayed by several years.
  • PoTS causes considerable disability – patients can become wheelchair or bed bound. They are often unable to continue education or employment (25%)
  • Treatments are available and 90% of patients will respond.
  • Patients frequently receive psychiatric labels prior to correct diagnosis. Tools used to assess anxiety commonly rely on somatic anxiety symptoms (which may reflect high upright catecholamine levels). Using the Anxiety Sensitive Index (a cognitive-based measure of anxiety), PoTS patients tend to have lower anxiety levels than the general population.

WHAT SHOULD I DO IF I SUSPECT PoTS?

  1. PoTS should be considered in patients (especially young women) with a combination of unexplained symptoms eg dizziness, syncope, fatigue, palpitations/tachycardia, headaches, exercise intolerance, anxiety
  2. Have a high index of suspicion in diabetes, chronic fatigue syndrome and joint hypermobility syndrome.
  3. Stand test – this can be undertaken in a GP consulting room.
  4. Exclude other pathology with biochemistry tests (see above) and ECG (in syncope or family history of sudden death, exclude prolonged QT interval).
  5. Consider 24 hour BP/ heart rate monitoring – normal recording does not exclude PoTS. (If equipment cannot be triggered by patient to record when symptomatic, PoTS, vasovagal syncope and orthostatic hypotension can be missed)
  6. If PoTS is suspected, referral should be made to a physician with an interest in PoTS as diagnosis and management can be challenging. These consultants are often cardiologists with an interest in arrhythmias (i.e. cardiac electrophysiologists who may work in a syncope or blackout clinic). There are also some neurologists, medicine for elderly and neurovascular (autonomic) consultants with an interest in PoTS. There is a list of doctors with an interest in PoTS and syncope on the PoTS UK and STARS websites. (see below)

www.potsuk.org

www.gpnotebook.co.uk

‘PoTS on a Page’ for GPs – one page A4 printable leaflet for GPs can be foundhere

Glossary for patients

arrhythmia – abnormal heart rhythm
auto-immune – when the immune system produces antibodies against the body’s own tissues
autonomic – belonging to the nervous system that controls bodily functions that we do not have to think about
catecholamine – a chemical produced by the adrenal gland
CBT -cognitive behavioural therapy, a talking therapy
cerebral perfusion – blood supply within the brain
deconditioning – lack of physical fitness
heterogeneous – varied or diverse
hypoperfusion -reduced flow of blood / fluids through tissues
hypotension – abnormally low blood pressure
hypovolaemia – low volume of circulating blood
inotropic – ability of muscles to contract, usually related to heart muscle
multi-system – affecting several organs in the body
neutrally mediated hypotension – low blood pressure due to abnormality of the autonomic nervous system
noradrenaline – is a type of catecholamine-see above
orthostatic intolerance – symptoms that develop in the upright position and are relieved by lying down
orthostatic tachycardia – increased heart rate due to becoming upright
physiological measures – lifestyle changes that do not involve medication
prolonged QT interval – ECG abnormality that can sometimes lead to serious abnormal heart rhythms
sympathetic – belonging to the autonomic nervous system and involved in the ‘fight or flight’ response
syncope – brief loss of consciousness due to reduced blood supply to the brain
vasoconstriction – narrowing of blood vessels
vasodilatation – dilatation of blood vessels
vaso-vagal syncope – transient loss of consciousness caused by an abnormal functioning of the autonomic (parasympathetic) nervous system
venous return -return of blood through blood vessels towards the heart

Written by Dr Lesley Kavi
Approved by Professor M D Gammage
Updated 1/4/2014
Review date 1/4/2016
Version 2

The First Question I Googled After the POTS Diagnosis

“What is the life expectancy for someone with POTS (postural orthostatic tachycardia syndrome)?”

This is what I inquired of Google when I received one of my first diagnoses. It was a simple question filled with fear. I didn’t want to know what the answer was, but my curiosity outweighed the fear.

I was immediately hit with a plethora of ambiguous answers that only added to my anxiety. Some professed your lifespan wasn’t altered by the diagnosis while others spoke of losing a family member at a grievous age. This opened up a greater question for me — was it worse to have a shorter lifespan or an average lifespan spent with a nasty syndrome?

I called my cardiologist the next day and nervously asked her the same question I’d asked Google. She laughed and told me my lifespan is not believed to be altered. This caused me to introspectively examine my future dreams and goals. This new diagnosis was going to present some obstacles in achieving them, that was for sure.

When my mom took me to get prayer at our church, our pastor, trying to be kind, said there were other ways to help people, discounting my hopes of wanting to become a doctor. It took me about a year of pondering and struggling before I realized no matter how many diagnoses I would receive, there was nothing that could stop me from achieving my goals. It would just be more rewarding for me in the long run, knowing I overcame the odds and still achieved goals I had since I was a child.

It was then I decided my diagnosis would not define my identity nor my future.

Postural Orthostatic Tachycardia Syndrome (POTS)

What is postural orthostatic tachycardia syndrome (POTS)?

Postural orthostatic tachycardia syndrome (POTS) is a condition that affects circulation (blood flow). It involves the autonomic nervous system (which automatically controls and regulates vital bodily functions) and sympathetic nervous system (which activates the fight or flight response).

POTS is a form of orthostatic intolerance, the development of symptoms that come on when standing up from a reclining position, and that may be relieved by sitting or lying back down. The primary symptom of an orthostatic intolerance is lightheadedness, fainting, and an uncomfortable, rapid increase in heartbeat.

Heart rate and blood pressure work together to keep the blood flowing at a healthy pace, no matter what position the body is in. People with POTS cannot coordinate the balancing act of blood vessel squeeze and heart rate response. This means the blood pressure cannot be kept steady and stable.

Each case of POTS is different. POTS patients may see symptoms come and go over a period of years. In most cases, with proper adjustments in diet, medications and physical activity, a person with POTS will see an improvement in quality of life. Also, if an underlying cause is found and is treated, POTS symptoms may subside.

There are various forms of POTS. The most common are:

  • Neuropathic POTS: Peripheral denervation (loss of nerve supply) leads to poor blood vessel muscles, especially in the legs and core body.
  • Hyperadrenergic POTS: Overactivity of the sympathetic nervous system
  • Low blood volume POTS: Reduced blood volume can lead to POTS. Low blood volume can cause similar symptoms that may overlap in neuropathic and hyperadrenergic POTS

Who is at risk for POTS?

The majority of POTS patients are women ages 13-50 years old. About 450,000 people suffer from POTS in the United States.

  • Patients may develop POTS after a viral illness, serious infections, medical illness, pregnancy, and trauma such as head injury. The condition may develop as aftermath of a significant illness (especially associated with hospitalization and prolonged immobilization).
  • POTS may develop in those who have had a recent history of mononucleosis.
  • People with certain autoimmune conditions such as Sjogren’s syndrome and celiac disease can be at higher risk. Sjogren’s can be evaluated by blood testing, dry eye test, lip biopsy, and rheumatology consult. Celiac disease can be tested through blood work, gastroenterology consult, and if needed biopsies of the small intestines

What are the symptoms of postural orthostatic tachycardia syndrome (POTS)?

POTS symptoms can be uncomfortable and frightening experiences. Patients with POTS usually suffer from two or more of the many symptoms listed below. Not all patients with POTS will have all these symptoms.

  • High/low blood pressure
  • High/low heart rate; racing heart rate
  • Chest pain
  • Dizziness/lightheadedness especially in standing up, prolonged standing in one position, or long walks
  • Fainting or near-fainting
  • Exhaustion/fatigue
  • Abdominal pain and bloating, nausea
  • Temperature deregulation (hot or cold)
  • Nervous, jittery feeling
  • Forgetfulness and trouble focusing (brain fog)
  • Blurred vision
  • Headaches and body pain/aches (may feel flu-like); neck pain
  • Insomnia and frequent awakenings from sleep, chest pain and racing heart rate during sleep, excessive sweating
  • Shakiness/tremors especially with adrenaline surges
  • Discoloration of feet and hands
  • Exercise intolerance
  • Excessive or lack of sweating
  • Diarrhea and/or constipation

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